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Case Reports
. 2022 Jan 24;2022(1):omab141.
doi: 10.1093/omcr/omab141. eCollection 2022 Jan.

Adams-Oliver syndrome, intestinal obstruction and heart defects: a case series of aplasia cutis congenita

Ihsan Baroudi  1 , Ola Alakhras  1 , Thaer Douri  2 , Nedal Alkhani  3
Affiliations
Case Reports

Adams-Oliver syndrome, intestinal obstruction and heart defects: a case series of aplasia cutis congenita

Ihsan Baroudi et al. Oxf Med Case Reports. .

Abstract

Aplasia cutis congenita (ACC) is a rare congenital defect described by the absence of skin and occasionally subcutaneous tissues or bone. The management of ACC varies depending on the lesion size, location and associated abnormalities. Small lesions often heal spontaneously, whereas larger lesions are significant and usually associated with additional anomalies in other organs. This paper reports three cases, which describe large lesions of ACC, presented with other abnormalities (Adams-Oliver syndrome, intestinal obstruction and heart defect). Particular attention should be paid to the patient with large lesions of ACC to investigate more congenital anomalies.

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Figures

Figure 1
Figure 1
(A&B) ACC appears as a hemorrhagic large defect extending over the occipital and parietal regions.
Figure 2
Figure 2
(A, B&C) Terminal limb anomalies; absence of toes, hypoplastic toes nails and additional dead finger in the hand.
Figure 3
Figure 3
Scar tissue on the abdomen.
Figure 4
Figure 4
The CT scan shows an absence of the skin and bone of the scalp.
Figure 5
Figure 5
(A&B) ACC appears as an extensive hemorrhagic lesion on the vertex of the scalp.
Figure 6
Figure 6
A nasogastric intubation was performed to treat the intestinal obstruction.
Figure 7
Figure 7
(A&B) A contrast barium enema presents intestinal obstruction.
Figure 8
Figure 8
ACC appears as a fibrotic alopecic scar on the vertex of the scalp.
Figure 9
Figure 9
An echocardiogram shows multiple malformations that include PFO, VSD, Tricuspid valve insufficiency and severe pulmonary valve stenosis.
See this image and copyright information in PMC

References

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